Head and Neck
Myxomas of the Rinaldo F. Canalis, MD; \s=b\
Myxomas
are
Geoffrey A. Smith, MD;
benign mesenchymal
tumors that occur rarely in the head and neck. When they do occur, they are prevalent in the jaws and occur less frequently in the subcutaneous tissues. We report ten new cases and review the clinical, structural, and behavioral characteristics of myxomas arising in various head and neck locations. All ages are affected. The most common initial complaint is the presence of a slow-growing mass. The sarcomas, especially liposarcomas and fibrosarcomas, rank high in the differential diagnosis of myxomas. These tumors are stubborn infiltrators. When a capsule is present, it is usually incomplete and insufficient to contain the tumor. Treatment is by wide surgical excision. The high recurrence rate previously reported is probably due to incomplete excision and is higher when enucleation or curettage are used. Long-term follow\x=req-\ up is mandatory since recurrences may develop several years after treatment.
(Arch Otolaryngol 102:300-305, 1976) of the head and neck in been literature. We the report ten new cases, review the clinical, structural, and behavioral characteristics of these tumors, and discuss their management.
Myxomas have rarely discussed otolaryngological HISTORICAL NOTE In
1871, Virchow1 used the
"myxomas"
tumors that
to describe
a
term group of
histologically resembled
the mucinous substance of the umbilical cord. Although he noted variations in the pattern of these growths, he recognized one type that remained a definite entity and did not differentiate into any other histologic variety. Unfortunately, Virchow's concept of for publication Jan 28, 1976. From the Division of Head and Neck Surgery, Department of Surgery, UCLA School of Medicine, Los Angeles, and the Division of Otolaryngology, Department of Surgery, Harbor General Hospital, Torrance, Calif. Reprint requests to the Division of Head and Neck Surgery, Department of Surgery, UCLA School of Medicine, 10833 Le Conte Ave, Los Angeles, CA 90024 (Dr Canalis).
Accepted
Horst R.
Konrad,
MD
myxoma as an independent tumor was lost in subsequent reports because various lesions that contain myxoid elements were erroneously labeled "myxomas" and "myxosarcomas." The need for clear diagnostic criteria was recognized by Stout,2 who presented the first extensive review of the subject in 1948. He defined myxomas as true neoplasms of mesenchymal origin and stated that these tumors do not metastasize and that their diagnosis excluded the presence of chondroblasts, lipoblasts, myoblasts, or other recognizable elements. Presently, Stout's definition is generally accepted; however, the existence of a malignant variant (myxosarcoma) is still controversial.3 MATERIAL
During the period 1955 to 1975, 25 myxomas arising in various anatomical sites were treated at the UCLA Center for the Health Sciences. Ten tumors of the head and neck were used as the material for this study. All ten specimens were reevaluated by the authors with the assistance of members of the Division of Surgical Pathology of the UCLA School of Medicine. Paraffin-preserved blocks were sectioned and stained with hematoxylineosin, Masson trichrome, PAS, and Southgate mucicarmine. The clinical features of these cases are summarized in the Table. All patients included had a basic metastatic workup and at least one-year's follow-up. Four representative cases recently treated by the Division of Head and Neck Surgery (Otolaryngology) are reported in detail. REPORT OF CASES Case 1
year-old
(Myxoma of man
was
the
seen
Maxilla).\p=m-\A32\x=req-\ by a surgeon
because of a Bell palsy. Radiograms taken at that time revealed a mass in the left maxillary sinus. The facial paralysis and six weeks later resolvedspontaneously, the patient underwent a Caldwell-Luc operation and biopsy of the tumor. The diagnosis was myxoma. Maxillectomy was recommended but declined. The patient
one year later, at which time a painless, firm, 2 \m=x\3-cm nonulcerated mass was noted at the lateral and anterior portions of the hard palate. The first and second molar teeth had been lost due to tumor growth. No trismus, infraorbital paresthesia, or vision loss were noted. The neck was free of masses. Plane radiograms of the sinuses (Fig 1) and tomograms of the middle part of the face (Fig 2) demonstrated a mass eroding the medial and lateral walls of the left maxillary antrum. Following biopsy and confirmation of the diagnosis, the patient underwent a maxillectomy with orbital preservation. At operation, the tumor appeared to have been resected with adequate margins (Fig 3); however, histologic sections showed tumor in the pterygoid region. This area was then reexplored, excised, and cauterized. After 12 months of treatment, there is no evidence of recurrence. The tumor consisted of a loose fibrillar and myxomatous matrix containing small spindle-shaped cells. The nuclei of these cells were regular and no mitotic activity was present. Special staining demonstrated contrast in collagen fibers, reticulin strands, and mucin. Although in certain areas the tumor appeared encapsulated, for the most part it was only incompletely contained and tumoral extension into the surrounding tissues was present. Case 2 (Myxoma of the Mandible).\p=m-\A14\x=req-\ year-old boy complained of pain in the right body of the mandible of four-months' duration. Physical examination revealed no abnormality, but radiographic studies revealed a 2 \m=x\2-cm, circumscribed radiolucent lesion in close proximity to a developing third molar tooth. A transoral biopsy
sought further advice
specimen was reported as cyst. Following extraction
a
keratinizing
of the second
and third molar teeth, a soft, glistening tumor was encountered. Enucleation and curettage of the lesion was initially performed. A frozen section of this tissue demonstrated a myxoma. It was then elected to extract the first molar tooth and to remove additional bone 1 cm beyond the original margin of resection. An iliac crest bone graft was needed to bridge the defect. The patient was maintained in intermaxillary fixation for six weeks. There was no evidence of recurrence 12 months after surgery.
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The tumor was formed by stellate and spindle-shaped cells loosely scattered in a myxoid matrix. The nuclei of these cells were regular and displayed no mitosis. Only a few vessels were noted, and the amount of collagen was relatively small. There was no capsule, and tumor was noted to extend into areas of bone, appearing grossly normal. Case 3 (Myxoma of the Parotid Region).-A 27-year-old man had a fourmonth history of a slowly enlarging indo¬ lent mass, just above the angle of the left side of the mandible. At examination, a soft, nontender, 3 x 3-cm ovoid mass appeared to arise from the lower pole of the left parotid gland. The facial nerve function was normal, and the neck was free of palpable masses. A parotid sialogram was normal. At operation, the tumor appeared to be firmly attached to the parotid gland (Fig 4). A frozen section done at the time of surgery was interpreted as a myxoma. The patient is tumor-free three years after superficial parotidectomy. The tumor was formed by uniform stel¬ late cells with ramifying cytoplasmic proc¬ a light mucinous esses contained in matrix. Sparse collagen fibers, fat, occa¬ sional vessels, and a few lymphocytes were present. There was no mitotic activity. The lesion originated in the tissues superficial to the parotid and invaded the fascia. The glandular tissue was tumor-free. Case 4 (Myxoma of the Soft Tissues of the Neck).-A 71-year-old man noted progressive enlargement of an otherwise asymptomatic neck mass that had been present for several years. Examination revealed a 2.5 x 2-cm, soft nontender mass in the midportion of the posterior area of the left side of the neck. At operation, the
tive tissue tumors composed of stel¬ late and spindle-shaped cells set in a sparse, finely reticular matrix (Fig 5). The degree of cellularity and the amount of collagen tissue present are variable. In some instances, the neo¬ plasm has a lobulated appearance and an
incomplete capsule.
The cause of myxomas is unknown. An infectious origin was suggested by Glazunov and Puchkov,' who demon¬ strated inclusion bodies in certain intramuscular myxomas. Trauma has occasionally been considered as a possible etiologic factor.'" In our group of patients, a history of previous injury was given in one case.
The histogenesis of myxomas is also unclear. Most authors-7 state that these tumors derive from primitive mesenchyme. Others7'·'1 believe that the myxomatous cell is an altered fibroblast secreting mucin in exces¬ sive amounts. Myxomas of the jaws may be histogenetically different from those arising elsewhere. Because osseous myxomas are exceptional out¬ side the jaws and often occur around erupting teeth, they are believed to have an odontogenic origin." Struc¬ tural studies demonstrating cellular islands similar to the reticulum of the enamel organ have strengthened this '"
hypothesis." Fig 1 .—Waters view in antrum.
case
1.
Although
myxomas
muscles,11
parotid gland,'-- phar¬
ades.
Myxomas may occur at any age, but prevalent in the third and fourth decades. Sexes are equally affected. They are usually isolated tumors; however, they may very rarely be multiple. One of our patients (Table, are
10) manifested three separate myxomas: one in the neck, a second in the upper arm, and a third in the thigh. In the head and neck, the most common initial complaint is the prèscase
Myxoma involving palate,
alveolar
tissues, and the trapezius muscle. The was excised with a wide of normal tissue. Thirty months after the second operation, the patient is
recurrent lesion
margin
tumor-free. The tumor
was very similar to the ones described. However, it was less cellular and had prominent collagen fibers. An incomplete, poorly organized fibrous capsule appeared to partially limit the original tumor. No capsule was present in the recurrence that was otherwise indistin¬ guishable from the primary lesion.
previously
COMMENT
benign
uncom¬
ynx,212' and larynx27'·2" have been reported during the past two dec¬
yellowish tumor that appeared to arise from the subcutaneous tissue. It was thought to be encapsulated and was shelled out. The histologie diagnosis was myxoma. Two months after resection, the tumor recurred, and at exploration, it was noted to involve the skin, subcutaneous
are
not
heart is well known, they are rare in the head and neck. When they occur in this region, they are prevalent in the jaws and to a lesser degree in the subcutaneous tissues. The only exten¬ sive series of extracardiac myxomas is the one by Stout.- He reviewed 143 lesions, 22 of which occurred in the head and neck. Eight of these arose in the jaws, five in the sinuses, four in the orbit, three in the pharynx, one in the parotid gland, and one in the carotid body. Among the myxomas involving the jaws, several short series totaling approximately 70 tu¬ mors have been discussed/'-" Myxo¬ mas of the tongue,1'1' preauricular region,17'1" nose,17"11' cheek,-"-1 neck
mass was a
The myxomas
are
mon, and their higher incidence in the
connec-
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ridge,
and left
maxillary
Clinical Characteristics of Ten Case/
Age, yr/Sex 1/32/M 2/14/M 3/27/M 4/71/M
Anatomic Location Maxilla Mandible Parotid Lateral part of neck
Size
Initial
Duration
(cm)
Complaint
(mo)
Myxomas of the Head
and Neck
Treatment Excision Excision Excision Enucleation
Recurrence No No No Once
Complications
Mass Pain Mass Mass
18
Unknown
Trauma No No No No
Mass
Unknown
No
Excision
No
None
1 X 0.5 2 X 0.7 1 X1
Mass Mass Mass
6
No No No
Excision Excision Excision
No No No
None None None
3X3 2X2
Mass Pain
Yes No
Excision Excision
No No
None Persistent
2X3 2.5 2 3X3 2.5 X 2
None Infection None None
Follow-Up (yr) 2V2
(subcutaneous) 5/46/F
Lateral part of neck
6/47/M 7/23/M 8/71/M
Nose Maxilla Chin
(muscle) Unknown
IV2
(subcutaneous) 9/2/M 10/39/F
Maxilla Lateral part of neck
18 24
pain
(subcutaneous)
encapsulated; however there is serious controversy as to the presence or absence of a capsule in these tumors. All of the tumors of our series were infiltrative. A rudimentary cap¬ sule was present in patients 1 and 4. Case 1 was interesting in that encap¬ sulation was evident only on the buccal surface, while where tumor extended into the maxillary antrum the lesion was retained only by the respiratory epithelium lining the si¬ nus (Fig 7). In the proximity of bone, the capsule exhibited multiple areas of tumoral breakthrough (Fig 8). Radiographically, myxomas involv¬ ing the viscerae of the head and neck will exhibit changes similar to those produced by other benign expanding lesions. Maxillary tumors will exhibit a variable degree of bone destruction. Mandibular myxomas may be unilocular, in no way discernible from isolated mandibular cysts, or multilocular, with a pattern similar to the strings of a tennis racket. Sonesson2* described these lesions as radiolucent defects divided by straight septae into
to be
Fig 2— Tomogram of middle part of face in walls of maxillary antrum.
case
of a soft indolent mass. Pain and loosening of involved teeth may occur in tumors of the jaws. Most tumors grow slowly, but occasionally they may enlarge rapidly and reach consid¬ erable size. One of the two extracar¬ diac myxomas known to have been lethal occurred in the neck of a 40enee
reported by Hogencompletely sur¬ rounded the trachea and esophagus, causing death by asphyxia. In our year-old
woman
auer.27 The tumor
1. Note destruction of medial and lateral
group of
patients, the average dura¬ tion of symptoms was ten months, and the average size of the lesion was 2
2
cm.
The gross aspect of these tumors is that of a slimy, grayish gelatinous mass. Their consistency varies from soft to firm, depending on their fibrous tissue content. The lesion may be infiltrated by fat, acquire a yellow¬ ish tinge, and resemble a lipomatous tumor
(Fig 6). Myxomas
may appear
square, rectangular, or triangular spaces, with a finely trabeculated central portion (Fig 9 and 10). Myxomas are to be differentiated
from sarcomas, mesenchymomas, and other malignant tumors in which areas of myxomatous degeneration is a frequent feature. The most common differential diagnosis is with liposarcoma (Fig 6). In our review of myxo¬ matous lesions at the UCLA Medical Center, there were six cases initially
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Fig 3.—Lateral aspect of maxillectomy specimen in patient 1. T, Tumor protruding
into antrum. C, Canine tooth. Arrow is at
posterior margin, apparently tumor-free, but histologically positive.
diagnosed as myxomas that with further study demonstrated to be three liposarcomas, two fibrosarcomas, and one chondrosarcoma. Myxo¬ mas must also be separated from other benign connective tissue lesions such as fibromas, fibroxanthomas, fibrous dysplasia, and nodular fasciitis. The existence of a malignant vari¬ ety of myxomas is controversial. Most authors agree with Stout and Lattes,7 who in a review of 205 soft tissue myxomas opposed the term "myxosarcoma" because they never found a tumor that metastasized. Their opin¬ ion has only once been seriously chal¬
lenged: Sponsel et al,' studying a group of 50 possible myxomas, were able to predict a benign or malignant behavior on the basis of the arrange¬ ment of the fibers and the vascular pattern.
For treatment purposes, myxomas to be regarded as benign infiltrative lesions with a strong tendency to recur locally. In the head and neck, the incidence of recurrences is higher in mandibular tumors, in which Zimmer¬ man and Dahlin" have noted a rate approaching 25%. Elsewhere in this region, examples of multiple recur¬ rences during a period of many years Recurrences may oc¬ are common.2 cur in periods varying from three months to ten years, but most will appear within the first two years after treatment. Among our patients, seven have been observed for two years or more after surgery. Only one recurrence has been recorded (case are
Fig 4.—Surgical specimen
area of
in patient 3. P, Parotid tissue. M, attachment of tumor to parotid fascia.
"'
4).
The
likely explanation for the high rate reported in the litera¬ is incomplete surgical excision.
recurrence
ture
Fig 5.—Representative section of myxoma (patient 4). Cells are set in sparse finely reticular stroma (hematoxylin-eosin, x80). Inset, Greater detail of stellate and spindle-shaped cells characteristic of these tumors (hematoxylin-eosin, 300).
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Myxoma. Arrow indicates
The surgeon is often misled by the apparent encapsulation of the lesion and enucleates it, leaving nests of viable tumor in the field. We have been impressed by the stubborn, infil-
trative nature of these lesions and by the frequency with which apparently normal tissue harbors tumoral cells. In superficial myxomas, excision with a margin usually presents no difficul-
ties. In tumors of complex anatomic location, orientation of the specimen with the pathologist and multiple operative frozen sections are of value to assure complete removal. In maxil¬ lary tumors, a partial maxillectomy is usually required to encompass the lesion. In the mandible, resection of the involved segment with immediate bridging of the defect is the method of choice. Myxomas developing in or about the
parotid gland may present special problems of management. As in case 3 described herein, it may be impossible to ascertain clinically, radiographically, or even surgically if the tumor involves the parotid gland or not. In
Fig 6.—Excellent demonstration of tendency of some subcutaneous myxomas to infiltrate (case 4). Differential diagnosis with liposarcomas may prove difficult (hematoxylineosin, x120). fat
such cases, the method of choice is excision by superficial parotidectomy. Successful resection of a preauricular mass by simple excision has been reported,1" and may be acceptable in selected cases; however, incomplete removal may lead to disastrous results. Dutz and Stout'" have re¬ ported the case of a 13-year-old girl who, after incomplete excision and irradiation of a parotid myxoma, required a resection of the remaining
Fig 7.—Myxoma invading maxillary antrum (case 1). Tumor lacks capsule and only by respiratory epithelium lining sinus (hematoxylin-eosin, 300). Fig 8.—Striking demonstration original magnification 120).
of tumoral invasion of bone in case 1
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is limited
(hematoxylin-eosin,
Fig 9.—Radiogram
bicuspid
tooth
of
(2B) to
large multilocular myxoma of mandible extending from second third molar (3M). T, Center of tumor.
Fig 10.—Closer view of tumor shown
2B, Second bicuspid tooth.
in
Fig
9. Note characteristic trabeculation of lesion.
Myxoma and myxosarcoma of the soft tissue of the extremities. J Bone Joint Surg 34:820-826, 1952. 4. Glazunov MF, Puchkov JG: Human muscular myxoma and intracellular inclusions. Vopr Onkol 6:11-27, 1960. 5. Enzinger FM: Intramuscular myxoma. Am J Clin Pathol 43:104-113, 1965. 6. Whitman RA, Stewart S, Stopack JG, et al: Myxoma of the mandible: Report of a case. J Oral Surg 28:63-70, 1971. 7. Stout AP, Lattes R: Atlas of tumor Pathology; Tumors of the soft tissue, in Atlas of Tumor Pathology. Washington, DC, Armed Forces Institute of Pathology, 1967, second series, pt 1, pp 35\x=req-\ 37. 8. Willis RA: Pathology of Tumors. St. Louis, CV Mosby Co, 1948, pp 642-655. 9. Zimmerman DC, Dahlin DC: Myxomatous tumors of the jaws. Oral Surg 11:1069-1080, 1958. 10. Barros RE, Dominguez FV, Cabrini RL: Myxoma of the jaws. Oral Surg 27:225-236, 1969. 11. Harrison JD: Odontogenic myxoma: Ultrastructural and histochemical studies. J Clin Pathol 26:570-582, 1973. 12. Ghosh BC, Huvos AG, Gerold FP, et al: Myxoma of the jaw bones. Cancer 31:237-240, 1973. 13. Bojanini NE: Mixoma de los maxilares. Antioquia Med 14:285-291, 1964. 14. Rosin RD: Intramuscular myxomas. Br J Surg 60:122-124, 1973. 15. Mincer R: Nerve sheath myxoma of the tongue. Oral Surg 37:428-430, 1974. 16. Bellinger CG: Preauricular myxoma. Plast Reconstr Surg 45:292-293, 1970. 17. Arslan, cited by Thompson S, Negus V: Diseases of the Nose and Throat, ed 5. New York, Appleton-Century-Crofts, Inc, 1943, p 345. 18. Faccini JM, Williams JL: Myxoma involving the soft tissues of the face. J Laryngol Otol 87:817-822, 1973. 19. Bochetto J, Minkowitz F, Minkowitz S, et al: Antral fibromyxoma presenting as a giant nasal polyp. Oral Surg 23:201-206, 1967. 20. Spengos MN, Schow CE: Myxomas of the soft tissues. J Oral Surg 23:140-144, 1965. 21. Louvel R: Mixoma benigno de la mejilla: Comentario de un caso. Prensa Med Arg 44:3083\x=req-\ 3086, 1957. 22. Malfatti T: Considerations on a case of myxoma of the parotid. Clin Pediatr 43:747-752,
gland, the facial nerve, and tissues of the subpharyngeal space to control the tumor.
Occasionally, radiotherapy has been as the primary mode of treat¬
used
ment for myxomas. Short-term
con¬
trol of these tumors by this method has been reported"1'1; however, no study exists demonstrating long-term cures by radiotherapy alone.
Juan Lechago, MD, prepared and interpreted histologie specimens. Paul H. Ward, MD, contrib¬ uted to the study of patient 3. Figures 9 and 10 were provided by the Section of Oral Radiology, UCLA School of Dentistry.
References 1. Virchow R: Die Cellularpathologie in ihrer Begrundung auf physiologische und patholo-
gische Gewebelehre. Berlin, Germany, Verlag von August Hirschwald, 1871, p 563. 2. Stout AP: Myxoma: The tumor of primitive mesenchyme. Ann Surg 127:706-719, 1948. 3. Sponsel KH, McDonald JR, Ghromley RK:
1961. 23. Babbitt JA, Pfeiffer DB: Myxoma of the palate and pharynx: Report of a case. Arch Otolaryngol 26:453-458, 1937. 24. Sinha SN, Yadav YC: Myxoma of the pharynx. Arch Otolaryngol 91:82-83, 1970. 25. Malfatti T: Contributions to the study of myxoma of the larynx. Minerva Otolaringol 11:395-402, 1961. 26. Usmanova NF: A rare case of laryngeal myxoma. Zh Ushn Nos Gorl Bolezn 28:105-106, 1968. 27. Hogenauer F: Uber einer Fall Kompletter Tracheal compression durch ein Myxoma des Halsen Zentrabl. J Chir 60:318-320, 1933. 28. Sonesson A: Odontogenio cysts and cystic tumors of the jaws: Roentgen-diagnostic and patho-anatomic study. Acta Radiol 81:104-114, 1950. 29. Trabucco A: Un caso di Mixoma della regione laterale del collo recidivato otto volte in 35 Anni. Minerva Med 2:351-353, 1933. 30. Dutz W, Stout AP: The myxoma in childhood. Cancer 14:629-635, 1961. 31. Attie JN, Catania A, Brenner S: Myxoma of the maxilla: Preoperative irradiation to facilitate dissection. Am J Roentgenol 96:19-24, 1966.
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Myxomas of the Rinaldo F. Canalis, MD; \s=b\
Myxomas
are
Geoffrey A. Smith, MD;
benign mesenchymal
tumors that occur rarely in the head and neck. When they do occur, they are prevalent in the jaws and occur less frequently in the subcutaneous tissues. We report ten new cases and review the clinical, structural, and behavioral characteristics of myxomas arising in various head and neck locations. All ages are affected. The most common initial complaint is the presence of a slow-growing mass. The sarcomas, especially liposarcomas and fibrosarcomas, rank high in the differential diagnosis of myxomas. These tumors are stubborn infiltrators. When a capsule is present, it is usually incomplete and insufficient to contain the tumor. Treatment is by wide surgical excision. The high recurrence rate previously reported is probably due to incomplete excision and is higher when enucleation or curettage are used. Long-term follow\x=req-\ up is mandatory since recurrences may develop several years after treatment.
(Arch Otolaryngol 102:300-305, 1976) of the head and neck in been literature. We the report ten new cases, review the clinical, structural, and behavioral characteristics of these tumors, and discuss their management.
Myxomas have rarely discussed otolaryngological HISTORICAL NOTE In
1871, Virchow1 used the
"myxomas"
tumors that
to describe
a
term group of
histologically resembled
the mucinous substance of the umbilical cord. Although he noted variations in the pattern of these growths, he recognized one type that remained a definite entity and did not differentiate into any other histologic variety. Unfortunately, Virchow's concept of for publication Jan 28, 1976. From the Division of Head and Neck Surgery, Department of Surgery, UCLA School of Medicine, Los Angeles, and the Division of Otolaryngology, Department of Surgery, Harbor General Hospital, Torrance, Calif. Reprint requests to the Division of Head and Neck Surgery, Department of Surgery, UCLA School of Medicine, 10833 Le Conte Ave, Los Angeles, CA 90024 (Dr Canalis).
Accepted
Horst R.
Konrad,
MD
myxoma as an independent tumor was lost in subsequent reports because various lesions that contain myxoid elements were erroneously labeled "myxomas" and "myxosarcomas." The need for clear diagnostic criteria was recognized by Stout,2 who presented the first extensive review of the subject in 1948. He defined myxomas as true neoplasms of mesenchymal origin and stated that these tumors do not metastasize and that their diagnosis excluded the presence of chondroblasts, lipoblasts, myoblasts, or other recognizable elements. Presently, Stout's definition is generally accepted; however, the existence of a malignant variant (myxosarcoma) is still controversial.3 MATERIAL
During the period 1955 to 1975, 25 myxomas arising in various anatomical sites were treated at the UCLA Center for the Health Sciences. Ten tumors of the head and neck were used as the material for this study. All ten specimens were reevaluated by the authors with the assistance of members of the Division of Surgical Pathology of the UCLA School of Medicine. Paraffin-preserved blocks were sectioned and stained with hematoxylineosin, Masson trichrome, PAS, and Southgate mucicarmine. The clinical features of these cases are summarized in the Table. All patients included had a basic metastatic workup and at least one-year's follow-up. Four representative cases recently treated by the Division of Head and Neck Surgery (Otolaryngology) are reported in detail. REPORT OF CASES Case 1
year-old
(Myxoma of man
was
the
seen
Maxilla).\p=m-\A32\x=req-\ by a surgeon
because of a Bell palsy. Radiograms taken at that time revealed a mass in the left maxillary sinus. The facial paralysis and six weeks later resolvedspontaneously, the patient underwent a Caldwell-Luc operation and biopsy of the tumor. The diagnosis was myxoma. Maxillectomy was recommended but declined. The patient
one year later, at which time a painless, firm, 2 \m=x\3-cm nonulcerated mass was noted at the lateral and anterior portions of the hard palate. The first and second molar teeth had been lost due to tumor growth. No trismus, infraorbital paresthesia, or vision loss were noted. The neck was free of masses. Plane radiograms of the sinuses (Fig 1) and tomograms of the middle part of the face (Fig 2) demonstrated a mass eroding the medial and lateral walls of the left maxillary antrum. Following biopsy and confirmation of the diagnosis, the patient underwent a maxillectomy with orbital preservation. At operation, the tumor appeared to have been resected with adequate margins (Fig 3); however, histologic sections showed tumor in the pterygoid region. This area was then reexplored, excised, and cauterized. After 12 months of treatment, there is no evidence of recurrence. The tumor consisted of a loose fibrillar and myxomatous matrix containing small spindle-shaped cells. The nuclei of these cells were regular and no mitotic activity was present. Special staining demonstrated contrast in collagen fibers, reticulin strands, and mucin. Although in certain areas the tumor appeared encapsulated, for the most part it was only incompletely contained and tumoral extension into the surrounding tissues was present. Case 2 (Myxoma of the Mandible).\p=m-\A14\x=req-\ year-old boy complained of pain in the right body of the mandible of four-months' duration. Physical examination revealed no abnormality, but radiographic studies revealed a 2 \m=x\2-cm, circumscribed radiolucent lesion in close proximity to a developing third molar tooth. A transoral biopsy
sought further advice
specimen was reported as cyst. Following extraction
a
keratinizing
of the second
and third molar teeth, a soft, glistening tumor was encountered. Enucleation and curettage of the lesion was initially performed. A frozen section of this tissue demonstrated a myxoma. It was then elected to extract the first molar tooth and to remove additional bone 1 cm beyond the original margin of resection. An iliac crest bone graft was needed to bridge the defect. The patient was maintained in intermaxillary fixation for six weeks. There was no evidence of recurrence 12 months after surgery.
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The tumor was formed by stellate and spindle-shaped cells loosely scattered in a myxoid matrix. The nuclei of these cells were regular and displayed no mitosis. Only a few vessels were noted, and the amount of collagen was relatively small. There was no capsule, and tumor was noted to extend into areas of bone, appearing grossly normal. Case 3 (Myxoma of the Parotid Region).-A 27-year-old man had a fourmonth history of a slowly enlarging indo¬ lent mass, just above the angle of the left side of the mandible. At examination, a soft, nontender, 3 x 3-cm ovoid mass appeared to arise from the lower pole of the left parotid gland. The facial nerve function was normal, and the neck was free of palpable masses. A parotid sialogram was normal. At operation, the tumor appeared to be firmly attached to the parotid gland (Fig 4). A frozen section done at the time of surgery was interpreted as a myxoma. The patient is tumor-free three years after superficial parotidectomy. The tumor was formed by uniform stel¬ late cells with ramifying cytoplasmic proc¬ a light mucinous esses contained in matrix. Sparse collagen fibers, fat, occa¬ sional vessels, and a few lymphocytes were present. There was no mitotic activity. The lesion originated in the tissues superficial to the parotid and invaded the fascia. The glandular tissue was tumor-free. Case 4 (Myxoma of the Soft Tissues of the Neck).-A 71-year-old man noted progressive enlargement of an otherwise asymptomatic neck mass that had been present for several years. Examination revealed a 2.5 x 2-cm, soft nontender mass in the midportion of the posterior area of the left side of the neck. At operation, the
tive tissue tumors composed of stel¬ late and spindle-shaped cells set in a sparse, finely reticular matrix (Fig 5). The degree of cellularity and the amount of collagen tissue present are variable. In some instances, the neo¬ plasm has a lobulated appearance and an
incomplete capsule.
The cause of myxomas is unknown. An infectious origin was suggested by Glazunov and Puchkov,' who demon¬ strated inclusion bodies in certain intramuscular myxomas. Trauma has occasionally been considered as a possible etiologic factor.'" In our group of patients, a history of previous injury was given in one case.
The histogenesis of myxomas is also unclear. Most authors-7 state that these tumors derive from primitive mesenchyme. Others7'·'1 believe that the myxomatous cell is an altered fibroblast secreting mucin in exces¬ sive amounts. Myxomas of the jaws may be histogenetically different from those arising elsewhere. Because osseous myxomas are exceptional out¬ side the jaws and often occur around erupting teeth, they are believed to have an odontogenic origin." Struc¬ tural studies demonstrating cellular islands similar to the reticulum of the enamel organ have strengthened this '"
hypothesis." Fig 1 .—Waters view in antrum.
case
1.
Although
myxomas
muscles,11
parotid gland,'-- phar¬
ades.
Myxomas may occur at any age, but prevalent in the third and fourth decades. Sexes are equally affected. They are usually isolated tumors; however, they may very rarely be multiple. One of our patients (Table, are
10) manifested three separate myxomas: one in the neck, a second in the upper arm, and a third in the thigh. In the head and neck, the most common initial complaint is the prèscase
Myxoma involving palate,
alveolar
tissues, and the trapezius muscle. The was excised with a wide of normal tissue. Thirty months after the second operation, the patient is
recurrent lesion
margin
tumor-free. The tumor
was very similar to the ones described. However, it was less cellular and had prominent collagen fibers. An incomplete, poorly organized fibrous capsule appeared to partially limit the original tumor. No capsule was present in the recurrence that was otherwise indistin¬ guishable from the primary lesion.
previously
COMMENT
benign
uncom¬
ynx,212' and larynx27'·2" have been reported during the past two dec¬
yellowish tumor that appeared to arise from the subcutaneous tissue. It was thought to be encapsulated and was shelled out. The histologie diagnosis was myxoma. Two months after resection, the tumor recurred, and at exploration, it was noted to involve the skin, subcutaneous
are
not
heart is well known, they are rare in the head and neck. When they occur in this region, they are prevalent in the jaws and to a lesser degree in the subcutaneous tissues. The only exten¬ sive series of extracardiac myxomas is the one by Stout.- He reviewed 143 lesions, 22 of which occurred in the head and neck. Eight of these arose in the jaws, five in the sinuses, four in the orbit, three in the pharynx, one in the parotid gland, and one in the carotid body. Among the myxomas involving the jaws, several short series totaling approximately 70 tu¬ mors have been discussed/'-" Myxo¬ mas of the tongue,1'1' preauricular region,17'1" nose,17"11' cheek,-"-1 neck
mass was a
The myxomas
are
mon, and their higher incidence in the
connec-
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ridge,
and left
maxillary
Clinical Characteristics of Ten Case/
Age, yr/Sex 1/32/M 2/14/M 3/27/M 4/71/M
Anatomic Location Maxilla Mandible Parotid Lateral part of neck
Size
Initial
Duration
(cm)
Complaint
(mo)
Myxomas of the Head
and Neck
Treatment Excision Excision Excision Enucleation
Recurrence No No No Once
Complications
Mass Pain Mass Mass
18
Unknown
Trauma No No No No
Mass
Unknown
No
Excision
No
None
1 X 0.5 2 X 0.7 1 X1
Mass Mass Mass
6
No No No
Excision Excision Excision
No No No
None None None
3X3 2X2
Mass Pain
Yes No
Excision Excision
No No
None Persistent
2X3 2.5 2 3X3 2.5 X 2
None Infection None None
Follow-Up (yr) 2V2
(subcutaneous) 5/46/F
Lateral part of neck
6/47/M 7/23/M 8/71/M
Nose Maxilla Chin
(muscle) Unknown
IV2
(subcutaneous) 9/2/M 10/39/F
Maxilla Lateral part of neck
18 24
pain
(subcutaneous)
encapsulated; however there is serious controversy as to the presence or absence of a capsule in these tumors. All of the tumors of our series were infiltrative. A rudimentary cap¬ sule was present in patients 1 and 4. Case 1 was interesting in that encap¬ sulation was evident only on the buccal surface, while where tumor extended into the maxillary antrum the lesion was retained only by the respiratory epithelium lining the si¬ nus (Fig 7). In the proximity of bone, the capsule exhibited multiple areas of tumoral breakthrough (Fig 8). Radiographically, myxomas involv¬ ing the viscerae of the head and neck will exhibit changes similar to those produced by other benign expanding lesions. Maxillary tumors will exhibit a variable degree of bone destruction. Mandibular myxomas may be unilocular, in no way discernible from isolated mandibular cysts, or multilocular, with a pattern similar to the strings of a tennis racket. Sonesson2* described these lesions as radiolucent defects divided by straight septae into
to be
Fig 2— Tomogram of middle part of face in walls of maxillary antrum.
case
of a soft indolent mass. Pain and loosening of involved teeth may occur in tumors of the jaws. Most tumors grow slowly, but occasionally they may enlarge rapidly and reach consid¬ erable size. One of the two extracar¬ diac myxomas known to have been lethal occurred in the neck of a 40enee
reported by Hogencompletely sur¬ rounded the trachea and esophagus, causing death by asphyxia. In our year-old
woman
auer.27 The tumor
1. Note destruction of medial and lateral
group of
patients, the average dura¬ tion of symptoms was ten months, and the average size of the lesion was 2
2
cm.
The gross aspect of these tumors is that of a slimy, grayish gelatinous mass. Their consistency varies from soft to firm, depending on their fibrous tissue content. The lesion may be infiltrated by fat, acquire a yellow¬ ish tinge, and resemble a lipomatous tumor
(Fig 6). Myxomas
may appear
square, rectangular, or triangular spaces, with a finely trabeculated central portion (Fig 9 and 10). Myxomas are to be differentiated
from sarcomas, mesenchymomas, and other malignant tumors in which areas of myxomatous degeneration is a frequent feature. The most common differential diagnosis is with liposarcoma (Fig 6). In our review of myxo¬ matous lesions at the UCLA Medical Center, there were six cases initially
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Fig 3.—Lateral aspect of maxillectomy specimen in patient 1. T, Tumor protruding
into antrum. C, Canine tooth. Arrow is at
posterior margin, apparently tumor-free, but histologically positive.
diagnosed as myxomas that with further study demonstrated to be three liposarcomas, two fibrosarcomas, and one chondrosarcoma. Myxo¬ mas must also be separated from other benign connective tissue lesions such as fibromas, fibroxanthomas, fibrous dysplasia, and nodular fasciitis. The existence of a malignant vari¬ ety of myxomas is controversial. Most authors agree with Stout and Lattes,7 who in a review of 205 soft tissue myxomas opposed the term "myxosarcoma" because they never found a tumor that metastasized. Their opin¬ ion has only once been seriously chal¬
lenged: Sponsel et al,' studying a group of 50 possible myxomas, were able to predict a benign or malignant behavior on the basis of the arrange¬ ment of the fibers and the vascular pattern.
For treatment purposes, myxomas to be regarded as benign infiltrative lesions with a strong tendency to recur locally. In the head and neck, the incidence of recurrences is higher in mandibular tumors, in which Zimmer¬ man and Dahlin" have noted a rate approaching 25%. Elsewhere in this region, examples of multiple recur¬ rences during a period of many years Recurrences may oc¬ are common.2 cur in periods varying from three months to ten years, but most will appear within the first two years after treatment. Among our patients, seven have been observed for two years or more after surgery. Only one recurrence has been recorded (case are
Fig 4.—Surgical specimen
area of
in patient 3. P, Parotid tissue. M, attachment of tumor to parotid fascia.
"'
4).
The
likely explanation for the high rate reported in the litera¬ is incomplete surgical excision.
recurrence
ture
Fig 5.—Representative section of myxoma (patient 4). Cells are set in sparse finely reticular stroma (hematoxylin-eosin, x80). Inset, Greater detail of stellate and spindle-shaped cells characteristic of these tumors (hematoxylin-eosin, 300).
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Myxoma. Arrow indicates
The surgeon is often misled by the apparent encapsulation of the lesion and enucleates it, leaving nests of viable tumor in the field. We have been impressed by the stubborn, infil-
trative nature of these lesions and by the frequency with which apparently normal tissue harbors tumoral cells. In superficial myxomas, excision with a margin usually presents no difficul-
ties. In tumors of complex anatomic location, orientation of the specimen with the pathologist and multiple operative frozen sections are of value to assure complete removal. In maxil¬ lary tumors, a partial maxillectomy is usually required to encompass the lesion. In the mandible, resection of the involved segment with immediate bridging of the defect is the method of choice. Myxomas developing in or about the
parotid gland may present special problems of management. As in case 3 described herein, it may be impossible to ascertain clinically, radiographically, or even surgically if the tumor involves the parotid gland or not. In
Fig 6.—Excellent demonstration of tendency of some subcutaneous myxomas to infiltrate (case 4). Differential diagnosis with liposarcomas may prove difficult (hematoxylineosin, x120). fat
such cases, the method of choice is excision by superficial parotidectomy. Successful resection of a preauricular mass by simple excision has been reported,1" and may be acceptable in selected cases; however, incomplete removal may lead to disastrous results. Dutz and Stout'" have re¬ ported the case of a 13-year-old girl who, after incomplete excision and irradiation of a parotid myxoma, required a resection of the remaining
Fig 7.—Myxoma invading maxillary antrum (case 1). Tumor lacks capsule and only by respiratory epithelium lining sinus (hematoxylin-eosin, 300). Fig 8.—Striking demonstration original magnification 120).
of tumoral invasion of bone in case 1
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is limited
(hematoxylin-eosin,
Fig 9.—Radiogram
bicuspid
tooth
of
(2B) to
large multilocular myxoma of mandible extending from second third molar (3M). T, Center of tumor.
Fig 10.—Closer view of tumor shown
2B, Second bicuspid tooth.
in
Fig
9. Note characteristic trabeculation of lesion.
Myxoma and myxosarcoma of the soft tissue of the extremities. J Bone Joint Surg 34:820-826, 1952. 4. Glazunov MF, Puchkov JG: Human muscular myxoma and intracellular inclusions. Vopr Onkol 6:11-27, 1960. 5. Enzinger FM: Intramuscular myxoma. Am J Clin Pathol 43:104-113, 1965. 6. Whitman RA, Stewart S, Stopack JG, et al: Myxoma of the mandible: Report of a case. J Oral Surg 28:63-70, 1971. 7. Stout AP, Lattes R: Atlas of tumor Pathology; Tumors of the soft tissue, in Atlas of Tumor Pathology. Washington, DC, Armed Forces Institute of Pathology, 1967, second series, pt 1, pp 35\x=req-\ 37. 8. Willis RA: Pathology of Tumors. St. Louis, CV Mosby Co, 1948, pp 642-655. 9. Zimmerman DC, Dahlin DC: Myxomatous tumors of the jaws. Oral Surg 11:1069-1080, 1958. 10. Barros RE, Dominguez FV, Cabrini RL: Myxoma of the jaws. Oral Surg 27:225-236, 1969. 11. Harrison JD: Odontogenic myxoma: Ultrastructural and histochemical studies. J Clin Pathol 26:570-582, 1973. 12. Ghosh BC, Huvos AG, Gerold FP, et al: Myxoma of the jaw bones. Cancer 31:237-240, 1973. 13. Bojanini NE: Mixoma de los maxilares. Antioquia Med 14:285-291, 1964. 14. Rosin RD: Intramuscular myxomas. Br J Surg 60:122-124, 1973. 15. Mincer R: Nerve sheath myxoma of the tongue. Oral Surg 37:428-430, 1974. 16. Bellinger CG: Preauricular myxoma. Plast Reconstr Surg 45:292-293, 1970. 17. Arslan, cited by Thompson S, Negus V: Diseases of the Nose and Throat, ed 5. New York, Appleton-Century-Crofts, Inc, 1943, p 345. 18. Faccini JM, Williams JL: Myxoma involving the soft tissues of the face. J Laryngol Otol 87:817-822, 1973. 19. Bochetto J, Minkowitz F, Minkowitz S, et al: Antral fibromyxoma presenting as a giant nasal polyp. Oral Surg 23:201-206, 1967. 20. Spengos MN, Schow CE: Myxomas of the soft tissues. J Oral Surg 23:140-144, 1965. 21. Louvel R: Mixoma benigno de la mejilla: Comentario de un caso. Prensa Med Arg 44:3083\x=req-\ 3086, 1957. 22. Malfatti T: Considerations on a case of myxoma of the parotid. Clin Pediatr 43:747-752,
gland, the facial nerve, and tissues of the subpharyngeal space to control the tumor.
Occasionally, radiotherapy has been as the primary mode of treat¬
used
ment for myxomas. Short-term
con¬
trol of these tumors by this method has been reported"1'1; however, no study exists demonstrating long-term cures by radiotherapy alone.
Juan Lechago, MD, prepared and interpreted histologie specimens. Paul H. Ward, MD, contrib¬ uted to the study of patient 3. Figures 9 and 10 were provided by the Section of Oral Radiology, UCLA School of Dentistry.
References 1. Virchow R: Die Cellularpathologie in ihrer Begrundung auf physiologische und patholo-
gische Gewebelehre. Berlin, Germany, Verlag von August Hirschwald, 1871, p 563. 2. Stout AP: Myxoma: The tumor of primitive mesenchyme. Ann Surg 127:706-719, 1948. 3. Sponsel KH, McDonald JR, Ghromley RK:
1961. 23. Babbitt JA, Pfeiffer DB: Myxoma of the palate and pharynx: Report of a case. Arch Otolaryngol 26:453-458, 1937. 24. Sinha SN, Yadav YC: Myxoma of the pharynx. Arch Otolaryngol 91:82-83, 1970. 25. Malfatti T: Contributions to the study of myxoma of the larynx. Minerva Otolaringol 11:395-402, 1961. 26. Usmanova NF: A rare case of laryngeal myxoma. Zh Ushn Nos Gorl Bolezn 28:105-106, 1968. 27. Hogenauer F: Uber einer Fall Kompletter Tracheal compression durch ein Myxoma des Halsen Zentrabl. J Chir 60:318-320, 1933. 28. Sonesson A: Odontogenio cysts and cystic tumors of the jaws: Roentgen-diagnostic and patho-anatomic study. Acta Radiol 81:104-114, 1950. 29. Trabucco A: Un caso di Mixoma della regione laterale del collo recidivato otto volte in 35 Anni. Minerva Med 2:351-353, 1933. 30. Dutz W, Stout AP: The myxoma in childhood. Cancer 14:629-635, 1961. 31. Attie JN, Catania A, Brenner S: Myxoma of the maxilla: Preoperative irradiation to facilitate dissection. Am J Roentgenol 96:19-24, 1966.
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